Familial cases tend to recur after surgical resection and occur in more than one sites.

Genetics

Pheochromocytomas are mostly sporadic but can also be familial. Most commonly, familial cases of pheochromocytoma are caused by mutation in succinate dehydrogenase (SDH) subunit gene. Familial pheochromocytomas are all autosomal dominant inheritance. Genes involved in pathogenesis includes:

Familial cases tend to recur after surgical resection and occur in more than one sites.

Genetics

Pheochromocytomas are mostly sporadic but can also be familial. Most commonly, familial cases of pheochromocytoma are caused by mutation in succinate dehydrogenase (SDH) subunit gene. Familial pheochromocytomas are all autosomal dominant inheritance. Genes involved in pathogenesis includes:

NF1 tumor suppressor gene (NH-1)

RET proto-oncogene mutation

VHL gene mutation

TMEM127 tumor suppressor gene mutation

SDH subunit gene mutations (SDHB, SDHC, SDHD, SDHAF2, SDHA)

Risk Factors

Adrenal incidentaloma

Hypertension at young age (<20 years)

Drug-resistant hypertension

Unexplained cardiomyopathy and atrial fibrillation

Commonly Associated Conditions

von Hippel-Lindau disease

NF1

Multiple endocrine neoplasia

Sturge-Weber syndrome

Tuberous sclerosis

Renal artery stenosis

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